Salimov Asif, Akyol Umut, Cildir Bunyamin, Batu Ezgi Deniz, Ozen Seza
Department of Otolaryngology, Hacettepe University Faculty of Medicine, 06100, Sihhiye, Ankara, Turkey.
Department of Otolaryngology, Hacettepe University Faculty of Medicine, 06100, Sihhiye, Ankara, Turkey.
Int J Pediatr Otorhinolaryngol. 2019 Apr;119:185-192. doi: 10.1016/j.ijporl.2019.01.042. Epub 2019 Feb 1.
Familial Mediterranean fever (FMF) is the most common monogenic autoinflammatory disease worldwide. It usually has a childhood onset and is characterized with recurrent attacks with irregular intervals. Few studies have been performed to investigate hearing in FMF patients ran with various tests and showed different results. In this study, first time in the literature, we aimed to evaluate and compare auditory functions in pediatric FMF patients during the attack periods and attack-free periods to see the possible effects of autoinflammation that was caused by FMF attacks.
40 pediatric FMF patients (80 ears) enrolled in the study as study group and 21 matching (42 ears) healthy children as a control group. Blood samples were taken from patients who presented clinical attack symptoms. Control group and FMF patients that were in attack period underwent audiometric evaluation including pure tone audiometry (0.25, 0.5, 1, 2, 4, 8 kHz) and Distortion product otoaoustic emission (DPOAE) (1, 1.4, 2, 2.8, 4 kHz) test. The tests were repeated in attackfree period. Correlations between hearing results and C-reactive protein, erythrocyte sedimentation rate levels, duration of disease, age at disease onset, colchicine usage time, cumulative dosage of colchicine and the number of attacks in the last six months were studied.
Hearing thresholds of FMF patients, compared to control group, were found to be increased at most frequencies. Lower signal/noise ratios (SNR) were detected at most frequencies in the DPAOE test. In the attack period compared to attack-free period, hearing thresholds in the audiometry were found to be increased at some frequencies (p < 0.05). However, this was not supported by the DPOAE test. In correlation analysis, increased colchicine usage time and colchicine cumulative dosage were associated with decreased thresholds in audiogram and increased SNR values in DPOAE. Detailed statistical analyses of all parameters were included in the study.
Results demonstrated cochlear involvement in FMF patients. Acute changes in hearing thresholds in the attack period may be suggesting the effect of acute inflammation on cochlea. The difference between the audiometry and otoacoustic emission test results of FMF patients and healthy controls suggests the cumulative effect of recurrent inflammation attacks on cochlea. Positive affect of treatment of the disease with long term colchicine in hearing were also demonstrated.
家族性地中海热（FMF）是全球最常见的单基因自身炎症性疾病。它通常在儿童期发病，其特征是发作间隔不规律。很少有研究通过各种测试来调查FMF患者的听力，结果也各不相同。在本研究中，我们首次旨在评估和比较小儿FMF患者在发作期和非发作期的听觉功能，以观察FMF发作引起的自身炎症可能产生的影响。
40例小儿FMF患者（80耳）作为研究组纳入本研究，21例匹配的健康儿童（42耳）作为对照组。采集出现临床发作症状患者的血样。对照组和处于发作期的FMF患者接受听力评估，包括纯音听力测定（0.25、0.5、1、2、4、8kHz）和畸变产物耳声发射（DPOAE）（1、1.4、2、2.8、4kHz）测试。在非发作期重复进行这些测试。研究听力结果与C反应蛋白、红细胞沉降率水平、病程、发病年龄、秋水仙碱使用时间、秋水仙碱累积剂量以及过去六个月内发作次数之间的相关性。
与对照组相比，FMF患者在大多数频率下的听力阈值升高。在DPAOE测试中，大多数频率下检测到较低的信噪比（SNR）。与非发作期相比，发作期听力测定中某些频率的听力阈值升高（p<0.05）。然而，DPOAE测试未支持这一结果。在相关性分析中，秋水仙碱使用时间增加和秋水仙碱累积剂量与听力图中阈值降低以及DPOAE中SNR值增加相关。研究纳入了所有参数的详细统计分析。
结果表明FMF患者存在耳蜗受累。发作期听力阈值的急性变化可能提示急性炎症对耳蜗的影响。FMF患者与健康对照者听力测定和耳声发射测试结果的差异表明反复炎症发作对耳蜗的累积影响。长期使用秋水仙碱治疗该疾病对听力的积极影响也得到了证实。
